Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Apr;162(2):353-362.
doi: 10.1007/s11060-023-04296-5. Epub 2023 Mar 23.

Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR)

Andrew Roehrig  1 , Daniel J Indelicato  2 , Arnold C Paulino  3 , Ralph Ermoian  4 , William Hartsell  5 , John Perentesis  6 , Christine Hill-Kayser  7 , Jae Y Lee  8 , Nadia N Laack  9 , Victor Mangona  10 , Iain MacEwan  11 , Bree R Eaton  12 , Sara Gallotto  13 , Benjamin V M Bajaj  13 , Paul D Aridgides  14 , Torunn I Yock  13
Affiliations

Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR)

Andrew Roehrig et al. J Neurooncol. 2023 Apr.

Erratum in

Abstract

Purpose: Atypical teratoid/rhabdoid tumors (ATRT) of the central nervous system (CNS) are rare tumors with a poor prognosis and variable use of either focal or craniospinal (CSI) radiotherapy (RT). Outcomes on the prospective Pediatric Proton/Photon Consortium Registry (PPCR) were evaluated according to RT delivered.

Methods: Pediatric patients receiving RT were prospectively enrolled on PPCR to collect initial patient, disease, and treatment factors as well as provide follow-up for patient outcomes. All ATRT patients with evaluable data were included. Kaplan-Meier analyses with log-rank p-values and cox proportional hazards regression were performed.

Results: The PPCR ATRT cohort includes 68 evaluable ATRT patients (median age 2.6 years, range 0.71-15.40) from 2012 to 2021. Median follow-up was 40.8 months (range 3.4-107.7). Treatment included surgery (65% initial gross total resection or GTR), chemotherapy (60% with myeloablative therapy including stem cell rescue) and RT. For patients with M0 stage (n = 60), 50 (83%) had focal RT and 10 (17%) had CSI. Among patients with M + stage (n = 8), 3 had focal RT and 5 had CSI. Four-year overall survival (OS, n = 68) was 56% with no differences observed between M0 and M + stage patients (p = 0.848). Local Control (LC) at 4 years did not show a difference for lower primary dose (50-53.9 Gy) compared to ≥ 54 Gy (73.3% vs 74.7%, p = 0.83). For patients with M0 disease, four-year OS for focal RT was 54.6% and for CSI was 60% (Hazard Ratio 1.04, p = 0.95. Four-year event free survival (EFS) among M0 patients for focal RT was 45.6% and for CSI was 60% (Hazard Ratio 0.71, p = 0.519). For all patients, the 4-year OS comparing focal RT with CSI was 54.4% vs 60% respectively (p = 0.944), and the 4-year EFS for focal RT or CSI was 42.8% vs 51.4% respectively (p = 0.610).

Conclusion: The PPCR ATRT cohort found no differences in outcomes according to receipt of either higher primary dose or larger RT field (CSI). However, most patients were M0 and received focal RT. A lower primary dose (50.4 Gy), regardless of patient age, is appealing for further study as part of multi-modality therapy.

Keywords: ATRT; Atypical teratoid/rhabdoid tumor; Brain; Pediatric; Proton; Radiotherapy.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Ostrom QT, Price M, Ryan K, Edelson J, Neff C, Cioffi G et al (2022) CBTRUS statistical report: pediatric brain tumor foundation childhood and adolescent primary brain and other central nervous system tumors diagnosed in the United States in 2014–2018. Neuro Oncol 24(Suppl 3):1–38. https://doi.org/10.1093/neuonc/noac161 - DOI
    1. Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL et al (1998) Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 22(9):1083–1092 - DOI - PubMed
    1. Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA et al (2009) Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27(3):385–389. https://doi.org/10.1200/jco.2008.18.7724 - DOI - PubMed - PMC
    1. SIOP Abstracts (2021) Abstracts. Pediatr Blood Cancer 68(S5):e29349. https://doi.org/10.1002/pbc.29349 - DOI
    1. Bartelheim K, Nemes K, Seeringer A, Kerl K, Buechner J, Boos J et al (2016) Improved 6-year overall survival in AT/RT: results of the registry study Rhabdoid 2007. Cancer Med 5(8):1765–1775. https://doi.org/10.1002/cam4.741 - DOI - PubMed - PMC

LinkOut - more resources